SASDB69: E244K monomeric human Properdin (E244K Human Properdin, E244K FP)

基本情報

• 登録情報: データベース: SASBDB / ID: SASDB69

試料

E244K monomeric human Properdin

• E244K Human Properdin (protein), E244K FP, Homo sapiens

機能・相同性

分子機能:

cytoplasmic side of Golgi membrane / positive regulation of opsonization / Defective B3GALTL causes PpS / O-glycosylation of TSR domain-containing proteins / Alternative complement activation / Activation of C3 and C5 / complement activation, alternative pathway / 補体 / Regulation of Complement cascade / specific granule lumen / positive regulation of immune response / tertiary granule lumen / defense response to bacterium / 免疫応答 / 小胞体 / Neutrophil degranulation / extracellular space / extracellular region

ドメイン・相同性:

: / Thrombospondin type 1 repeat / Thrombospondin type 1 domain / Thrombospondin type-1 (TSP1) repeat superfamily / Thrombospondin type-1 (TSP1) repeat profile. / Thrombospondin type 1 repeats / Thrombospondin type-1 (TSP1) repeat

構成要素:

Properdin

• 生物種: Homo sapiens (ヒト)
• 引用: ジャーナル: EMBO J / 年: 2017; タイトル: Functional and structural insight into properdin control of complement alternative pathway amplification.; 著者: Dennis V Pedersen / Lubka Roumenina / Rasmus K Jensen / Trine Af Gadeberg / Chiara Marinozzi / Capucine Picard / Tania Rybkine / Steffen Thiel / Uffe Bs Sørensen / Cordula Stover / Veronique Fremeaux-Bacchi / Gregers R Andersen / ; 要旨: Properdin (FP) is an essential positive regulator of the complement alternative pathway (AP) providing stabilization of the C3 and C5 convertases, but its oligomeric nature challenges structural analysis. We describe here a novel FP deficiency (E244K) caused by a single point mutation which results in a very low level of AP activity. Recombinant FP E244K is monomeric, fails to support bacteriolysis, and binds weakly to C3 products. We compare this to a monomeric unit excised from oligomeric FP, which is also dysfunctional in bacteriolysis but binds the AP proconvertase, C3 convertase, C3 products and partially stabilizes the convertase. The crystal structure of such a FP-convertase complex suggests that the major contact between FP and the AP convertase is mediated by a single FP thrombospondin repeat and a small region in C3b. Small angle X-ray scattering indicates that FP E244K is trapped in a compact conformation preventing its oligomerization. Our studies demonstrate an essential role of FP oligomerization while our monomers enable detailed structural insight paving the way for novel modulators of complement.

登録者

• Rasmus Jensen (Aarhus University, Aarhus, Denmark)

モデル

• モデル #1099: ; タイプ: atomic / ソフトウェア: CORAL / ダミー原子の半径: 1.90 A / カイ2乗値: 1.006 / P-value: 0.609000; Omokage検索でこの集合体の類似形状データを探す (詳細)

試料

• 試料: 名称: E244K monomeric human Properdin / 試料濃度: 2.70-2.70
• バッファ: 名称: 10 mM HEPES 150 mM NaCl / pH: 7.2

要素 #565

名称: E244K FP / タイプ: protein / 記述: E244K Human Properdin / 分子量: 49.345 / 分子数: 1 / 由来: Homo sapiens / 参照: UniProt: P27918
配列:

DPVLCFTQYE ESSGKCKGLL GGGVSVEDCC LNTAFAYQKR SGGLCQPCRS PRWSLWSTWA PCSVTCSEGS QLRYRRCVGW NGQCSGKVAP GTLEWQLQAC EDQQCCPEMG GWSGWGPWEP CSVTCSKGTR TRRRACNHPA PKCGGHCPGQ AQESEACDTQ QVCPTHGAWA TWGPWTPCSA SCHGGPHEPK ETRSRKCSAP EPSQKPPGKP CPGLAYKQRR CTGLPPCPEN LYFQGVAGGW GPWGPVSPCP VTCGLGQTME QRTCNHPVPQ HGGPFCAGDA TRTHICNTAV PCPVDGEWDS WGEWSPCIRR NMKSISCQEI PGQQSRGRTC RGRKFDGHRC AGQQQDIRHC YSIQHCPLKG SWSEWSTWGL CMPPCGPNPT RARQRLCTPL LPKYPPTVSM VEGQGEKNVT FWGRPLPRCE ELQGQKLVVE EKRPCLHVPA CKDPEEEEL

実験情報

• ビーム: 設備名称: PETRA III P12 / 地域: Hamburg / 国: Germany / 線源: X-ray synchrotronシンクロトロン / 波長: 0.124 Å / スペクトロメータ・検出器間距離: 3 mm
• 検出器: 名称: Pilatus 2M

スキャン

タイトル: E244K monomeric human Properdin / 測定日: 2016年7月27日 / 保管温度: 20 °C / セル温度: 20 °C / 照射時間: 0.05 sec. / フレーム数: 20 / 単位: 1/nm /

	Min	Max
Q	0.1543	2.7991

結果

Experimental MW: 50 kDa / カーブのタイプ: single_conc

	Guinier	Guinier error
前方散乱 I0	16659	67.36
慣性半径, Rg	4.13 nm	0.04

	Min	Max
Guinier point	5	59